This study describes health‐related quality of life (HRQoL) of persons with haemophilia A in the United States (US) and determines associations between self‐reported joint pain, motion limitation and ...clinically evaluated joint range of motion (ROM), and between HRQoL and ROM. As part of a 2‐year cohort study, we collected baseline HRQoL using the SF‐12 (adults) and PedsQL (children), along with self‐ratings of joint pain and motion limitation, in persons with factor VIII deficiency recruited from six Haemophilia Treatment Centres (HTCs) in geographically diverse regions of the US. Clinically measured joint ROM measurements were collected from medical charts of a subset of participants. Adults (N = 156, mean age: 33.5 ± 12.6 years) had mean physical and mental component scores of 43.4 ± 10.7 and 50.9 ± 10.1, respectively. Children (N = 164, mean age: 9.7 ± 4.5 years) had mean total PedsQL, physical functioning, and psychosocial health scores of 85.9 ± 13.8, 89.5 ± 15.2, and 84.1 ± 15.3, respectively. Persons with more severe haemophilia and higher self‐reported joint pain and motion limitation had poorer scores, particularly in the physical aspects of HRQoL. In adults, significant correlations (P < 0.01) were found between ROM measures and both self‐reported measures. Except among those with severe disease, children and adults with haemophilia have HRQoL scores comparable with those of the healthy US population. The physical aspects of HRQoL in both adults and children with haemophilia A in the US decrease with increasing severity of illness. However, scores for mental aspects of HRQoL do not differ between severity groups. These findings are comparable with those from studies in European and Canadian haemophilia populations.
Background: Disrupted circadian coordination accelerates malignant growth, but the molecular mechanism is unclear. Methods: Healthy or Glasgow osteosarcoma–bearing mice (n = 162) were synchronized ...with light and darkness over 2–3 weeks, submitted to an 8-hour advance onset of light every 2 days (chronic jet lag) to disrupt circadian coordination, or submitted to chronic jet lag and meal timing to prevent molecular clock alteration. The expression of molecular clock genes and of the cell cycle genes c-Myc and p53 in liver and tumor was determined with quantitative reverse transcription–polymerase chain reaction at six circadian times over a 24-hour period of light and darkness and analyzed with analysis of variance and cosinor. Tumor weight was measured daily over the course of the experiment. All statistical tests were two-sided. Results: In synchronized mice, mean mRNA levels of clock genes Rev-erbα, Per2, and Bmal1 varied by 206-, four-, and 26-fold, respectively, over the 24 hours in healthy mouse liver; by 36-, 35-, and 32-fold in the livers of tumor-bearing mice; and by 9.4-, 5.5-, and sixfold in tumor tissue (P = .046 to <.001). In mice subjected to chronic jet lag, the periodic changes were dampened and the clock gene rhythms were temporally shifted in liver and ablated in tumor, and tumor growth was accelerated. Meal timing reversed the chronic jet lag–induced alterations in Rev-erbα and Per2 expression in liver and of all three clock genes in tumor and slowed tumor growth. Tumor growth differed as a function of light and feeding schedules (P = .04). No obvious rhythm was detected for p53 or c-Myc in liver or tumor tissues of synchronized mice. In healthy mice subjected to chronic jet lag, the mean level of p53 expression was cut in half (P = .002), and a 12-fold circadian variation in c-Myc mRNA level (P = .03) was induced in the liver of healthy mice, whereas complex expression patterns were found in the liver and tumor of tumor-bearing mice. Conclusions: Altered light–dark or feeding schedules modified the expression of molecular clock genes and genes involved in carcinogenesis and tumor progression.
► We evaluate the risks on soils under repeated cultivation of transgenic cottons. ► Cry1Ac and CpTI proteins contents are higher in rhizophere soils than that in bulk soils. ► CpTI inhibits the ...degradation of Cry1Ac in rhizosphere soils under Bt–CpTI cottons. ► Cry1Ac proteins adversely affect on soil microbial properties and enzyme activities.
We evaluated the effect of transgenic cottons (Bacillus thuringiensis (Bt) and Bt with cowpea trypsin inhibitor (Bt–CpTI)) on soil ecosystems after three and four years of growth. The persistence and the relationships among insecticidal crystal proteins 1Ac (Cry1Ac) and cowpea trypsin inhibitor (CpTI) proteins derived from transgenic cottons, microbial properties, and enzymatic activities in the rhizosphere soil were investigated in the repeated cultivation experiment. The Cry1Ac and CpTI proteins persisted in the rhizosphere soil of transgenic cotton, whereas no proteins were detected in the rhizosphere soil of non-transgenic cotton. The persistence of CpTI inhibited the degradation of Cry1Ac protein in the rhizosphere soil of Bt–CpTI cotton. Repeated measures analysis of variance indicated that the year of cultivation, the variety of cotton, the transgenic trait, and their interactions significantly influenced the microbial properties and the enzymatic activities in rhizosphere soil. Overall, the repeated cultivation of transgenic cotton had significantly negative effects on the microbial properties and the enzymatic activities in rhizosphere soil compared to those in the rhizosphere soil of non-transgenic cotton.
The dynamic response of structures in contact with soil is receiving increasing interest and there is a growing need for more accurate models capable of simulating the behaviour of these systems. ...This is particularly important in the field of offshore wind turbines, where accurate estimates of system frequency are needed to avoid resonance, and in the structural health monitoring fields, where accurate reference damage models are used. Previous work has shown that there is significant uncertainty in how to specify mobilised soil stiffness for dynamic soil-pile interaction modelling. Moreover, the contribution of soil mass in dynamic motion is often ignored. This paper applies a finite-element iterative model updating approach previously developed by the authors to two experimental piles to ascertain the mobilised soil stiffness and mass profiles from impact test data. The method works by obtaining a frequency response function (FRF) from an impact test performed on a test pile, developing a numerical model of this system, applying initial estimates of soil mass and stiffness, and updating these properties to match the experimental FRF with that generated in the numerical model. A range of elements are investigated including multiple runs of the approach to test repeatability, the influence of different starting estimates for stiffness, the effect of variability in experimental test data, and the influence of the pile length over which masses are distributed. Moreover, potential sources of error are discussed. The method provides reasonably consistent estimates of the soil stiffness and mass acting in the lateral dynamic motion of a given pile tested in this paper. The approach may be useful in the continued improvement of Soil-Structure Interaction (SSI) modelling for dynamic applications.
•Paper investigates FE model updating approach applied to two test piles.•Soil stiffness is initially estimated using subgrade reaction approaches.•FRFs are developed from the test piles using impact testing.•FRFs are used in the FE updating approach to estimate acting stiffness and mass.•Sources of error are investigated and discussed.
Abstract
Electron-scale magnetic holes filled with high-energy electrons can provide a seed population of electrons in the magnetosphere and might play an important role in the interaction between ...the magnetosphere and solar wind. Theoretical studies have investigated their generation mechanisms based on the 1D or 2D geometry of the structure. However, the generation mechanism is still unclear. Here we report on the 3D geometry of the electron-scale magnetic hole in the solar wind based on the Magnetospheric Multiscale mission. We find that the cross section of the magnetic hole with a size of ∼0.2–0.6
ρ
i
(ion gyroradius) is either circular or 2D sheet-like. Electron vortices exist in both kinds of cross sections. The ellipse is a possible candidate for the geometry of the magnetic hole in the plane including its axis. Surprisingly, such an elliptical geometry suggests that the axial lengths of all our selected magnetic holes are ∼1–2
ρ
i
. This 3D geometry might shed some light on the generation mechanism and role of the electron-scale magnetic hole in the astrophysical plasma.
Colloidal chemistry holds promise to prepare uniform and size-controllable pre-catalysts; however, it remains a challenge to unveil the atomic-level transition from pre-catalysts to active catalytic ...surfaces under the reaction conditions to enable the mechanistic design of catalysts. Here, we report an ambient-pressure X-ray photoelectron spectroscopy study, coupled with in situ environmental transmission electron microscopy, infrared spectroscopy, and theoretical calculations, to elucidate the surface catalytic sites of colloidal Ni nanoparticles for CO2 hydrogenation. We show that Ni nanoparticles with phosphine ligands exhibit a distinct surface evolution compared with amine-capped ones, owing to the diffusion of P under oxidative (air) or reductive (CO2 + H2) gaseous environments at elevated temperatures. The resulting NiP x surface leads to a substantially improved selectivity for CO production, in contrast to the metallic Ni, which favors CH4. The further elimination of surface metallic Ni sites by designing multi-step P incorporation achieves unit selectivity of CO in high-rate CO2 hydrogenation.
To better understand the clinical characteristics of pulmonary nocardiosis associated with bronchiectasis.
Patients diagnosed as bronchiectasis complicated with pulmonary nocardiosis in 9 tertiary ...general hospitals in China were enrolled from March 2016 to March 2020, with the record of general data, imaging performance and pathogen. The literature was reviewed.
Totally 17 patients were included. There were 12 females and 5 males. The ages ranged from 45 to 79 years, with an average of (63±9) years. There were 15 nonsmokers and 2 smokers, all of whom with chronic course. The clinical manifestations were mostly cough, expectoration, hemoptysis, fever, and dyspnea. The imaging manifestation was bronchiectasis in both lungs, with the most common involvement in the left lower lung, right middle lobe and left lingual lobe. Sputum cultures were positive in 10 cases, bronchoalveolar lavage fluid (BALF) cultures were positive in 6 cases, and next generation gene sequencings were positive in 4 cases, including 2 cases
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